FH
fumarate hydratase
Synonyms:
HLRCC, LRCC, MCL, MCUL1
Species:
Human FH (2271), Mouse fh (14193), Rat Fh (24368), Thale Cress FH (828013), Horse FH (100060381), domestic cat FH (101096029), dog FH (480092), chicken FH (420969), Zebrafish fh (393938), fruit fly fh (31845) ...More
UniProtKB ID:
Gene ID:
- Human(2271) Summary: The protein encoded by this gene is an enzymatic component of the tricarboxylic acid (TCA) cycle, or Krebs cycle, and catalyzes the formation of L-malate from fumarate. It exists in both a cytosolic form and an N-terminal extended form, differing only in the translation start site used. The N-terminal extended form is targeted to the mitochondrion, where the removal of the extension generates the same form as in the cytoplasm. It is similar to some thermostable class II fumarases and functions as a homotetramer. Mutations in this gene can cause fumarase deficiency and lead to progressive encephalopathy. [provided by RefSeq, Jul 2008]
- Mouse(14193) fetal hematoma
- Rat(24368) fumarate hydratase
- Thale Cress(828013) frataxin
- Horse(100060381) fumarate hydratase
- domestic cat(101096029) fumarate hydratase
- dog(480092) fumarate hydratase
- chicken(420969) fumarate hydratase
- Zebrafish(393938) fumarate hydratase
- fruit fly(31845) frataxin homolog
- naked mole-rat(101715373) fumarate hydratase
- domestic guinea pig(100723287) fumarate hydratase
- sheep(100302102) fumarate hydratase
- cow(520260) fumarate hydratase
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